OBJECTIVE: An overview of the types of uterine sarcoma, supplemented by an illustrative case report of a rare uterine childhood tumor. CASE REPORT: Our case report describes the case of a 9-year-old girl with a 15-cm tumor in the abdominal cavity, which prolapsed vaginally before introitus during a clinical examination of the abdominal wall causing life-threatening bleeding. This bleeding was stopped by ligation of the tumor pedicle and its resection. Histological examination of the resected specimen described Müllerian adenosarcoma of the uterus. Based on the staging, laparoscopic hysterectomy with bilateral salpingectomy was indicated by the oncogynecological committee. The operation was performed without complications, and intraoperatively, a complete inversion of the uterus was diagnosed.

• Klíčová slova
• Leiomyosarcoma, Müllerian adenosarcoma, endometrial stromal sarcoma, leiomyosarcoma, uterine inversion, uterine sarcoma,
• MeSH
• adenosarkom * komplikace   chirurgie   patologie   diagnóza   MeSH
• dítě MeSH
• hysterektomie MeSH
• inverze dělohy * etiologie   MeSH
• lidé MeSH
• nádory dělohy * komplikace   chirurgie   patologie   diagnóza   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

OBJECTIVE: Uterine sarcomas are a rare and heterogeneous group of malignancies that include different histological sub-types. The aim of this study was to identify and evaluate the impact of the different prognostic factors on overall survival and disease-free survival of patients with uterine sarcoma. METHODS: This international multicenter retrospective study included 683 patients diagnosed with uterine sarcoma at 46 different institutions between January 2001 and December 2007. RESULTS: The 5-year overall survival for leiomyosarcoma, endometrial stromal sarcoma, undifferentiated sarcoma, and adenosarcoma was 65.3%, 78.3%, 52.4%, and 89.5%, respectively, and the 5-year disease-free survival was 54.3%, 68.1%, 40.3%, and 85.3%, respectively. The 10-year overall survival for leiomyosarcoma, endometrial stromal sarcoma, undifferentiated sarcoma and adenosarcoma was 52.6%, 64.8%, 52.4%, and 79.5%, respectively, and the 10-year disease-free survival was 44.7%, 53.3%, 40.3%, and 77.5%, respectively. The most significant factor associated with overall survival in all types of sarcoma except for adenosarcoma was the presence of residual disease after primary treatment. In adenosarcoma, disease stage at diagnosis was the most important factor (hazard ratio 17.7; 95% CI 2.86 to 109.93). CONCLUSION: Incomplete cytoreduction, tumor persistence, advanced stage, extra-uterine and tumor margin involvement, and the presence of necrosis were relevant prognostic factors significantly affecting overall survival in uterine sarcoma. The presence of lymph vascular space involvement and administration of adjuvant chemotherapy were significantly associated with a higher risk of relapse.

• Klíčová slova
• sarcoma, uterine cancer,
• MeSH
• adenosarkom * terapie   patologie   MeSH
• endometriální stromální sarkom * terapie   patologie   MeSH
• leiomyosarkom * patologie   MeSH
• lidé MeSH
• lokální recidiva nádoru MeSH
• nádory dělohy * patologie   MeSH
• nádory endometria * patologie   MeSH
• nádory pánve * MeSH
• prognóza MeSH
• retrospektivní studie MeSH
• sarkom * diagnóza   MeSH
• Check Tag
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

A case of gastrointestinal stromal tumor (GIST) with an unusual glandular component is reported. The tumor was found in the gastric fundus of a 93-year-old woman. Histologically, the lesion showed a biphasic adenosarcoma-like structure. Typical low-grade spindle cell patterns of GIST were intermingled with numerous and partly cystic glands. The glandular epithelium had pyloric/foveolar-like appearance, with foci of intestinal metaplasia and low-grade dysplasia. The stromal component was immunoreactive for CD117 (c-kit) and CD34, and negative for myoid and neuroid markers. The ultrastructural examination found nondescript and undifferentiated spindle cells. The gastric mucosa and submucosa near the tumor contained a small area with features of gastritis cystica profunda, with glands similar to those present inside the tumor. Therefore, a collision of GIST and gastritis cystica profunda is suggested in the histogenesis of the lesion.

• MeSH
• adenosarkom patologie   MeSH
• gastrointestinální stromální tumory patologie   MeSH
• lidé MeSH
• nádory žaludku patologie   MeSH
• senioři nad 80 let MeSH
• Check Tag
• lidé MeSH
• senioři nad 80 let MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

BACKGROUND: Uterine adenosarcoma is a rarely by occurring tumor. It is composed of a benign adenoid structure and a sarcomatous stromal component. The average age of patients with a diagnosis of uterine adenosarcoma is about 70 years. CASE: We present a case of a 19-year-old woman with a rarely occurring uterine adenosarcoma manifesting itself by irregular bleeding and producing fragile polypous matter which was spreading into the vagina. The final diagnosis was made only by repeated biopsies. Abdominal hysterectomy with bilateral salpingo-oophorectomy, appendectomy and revision of iliac lymph nodes were performed. Teleradiotherapy was applied from 4 fields in 25 fractions to a total exposure of 50 Gy. It was followed by six cycles of chemotherapy containing 50 mg/m2 doxorubicin and 5 g/m2 ifosfamid administered in 21-day dose intervals. CONCLUSION: This case should demonstrate the difficulty of making the right diagnosis. Since the end of therapy the patient has been regularly seen in our onco-gynecologic department. Now, 40 months after the end of chemotherapy and 46 months after making the diagnosis, there are no signs of relapse.

• MeSH
• adenosarkom patologie   chirurgie   MeSH
• dospělí MeSH
• hysterektomie MeSH
• lidé MeSH
• nádory dělohy patologie   chirurgie   MeSH
• následné studie MeSH
• přežití bez známek nemoci MeSH
• uterus patologie   MeSH
• vagina patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

• MeSH
• adenosarkom * diagnóza   patologie   chirurgie   MeSH
• dospělí MeSH
• lidé MeSH
• nádory dělohy * diagnóza   patologie   chirurgie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

• Klíčová slova
• NEPHROBLASTOMA/in infant and child *,
• MeSH
• adenosarkom * MeSH
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• Wilmsův nádor * MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH